Multimodality Treatment Outcome in Adult Patients with Head and Neck Rhabdomyosarcoma.

Objective(s): Head and neck rhabdomyosarcoma (HNRMS) is a rare malignant tumor in adults. No standard treatment for adults with HNRMS currently exists.

Methods: A retrospective study of 72 newly diagnosed consecutive adult patients with HNRMS was conducted at one institution between November 2010 and April 2023.

Synthetic inhibition of SREBP2 and the mevalonate pathway blocks rhabdomyosarcoma tumor growth in vitro and in vivo and promotes chemosensitization.

By analyzing RNA datasets from rhabdomyosarcoma (RMS), a soft tissue tumor with a prevalence in young people, we found upregulation of sterol regulatory element-binding protein 2 (SREBP2) and mevalonate pathway (MVP) genes, including 3-Hydroxy-3-Methylglutaryl-CoA Reductase (HMGCR), farnesyl-diphosphate synthase (FDPS), squalene epoxidase (SQLE), which correlated with worse overall patient survival and predicted statin sensitivity. In human RD and RH30 lines, treatment with 0.01-1 μM doses of fatostatin (SREBP2 inhibitor), lovastatin and simvastatin (HMGCR inhibitors), and zoledronic acid (FDPS inhibitor) impaired cell growth and migration, which were conversely stimulated by 50-100 μM cholesterol (CHO) supplementation.

Primary Esophageal Rhabdomyosarcoma: An Exceptionally Rare Cause of Pediatric Dysphagia.

Esophageal embryonal rhabdomyosarcoma (ERMS), a rare pediatric cancer, mimicked achalasia in a case involving dysphagia and vomiting. Diagnosis and chemotherapy necessitate careful monitoring due to potential complications. A 12-year-old girl with no prior medical history presented with progressive dysphagia and vomiting.

The childhood cancer data initiative: enabling data sharing to drive research advances and transform pediatric cancer diagnosis and treatment.

Purpose Of Review: With growing emphasis on data-driven research in pediatric oncology, particularly in the context of advances in molecular characterization and precision medicine, there is an urgent need for comprehensive data-sharing initiatives. This review explores how the Childhood Cancer Data Initiative (CCDI) addresses this critical need.

Recent Findings: CCDI plays a key role in enhancing pediatric cancer research by improving data integration, sharing, and collaboration.

Tumor-derived G-CSF induces an immunosuppressive microenvironment in an osteosarcoma model, reducing response to CAR.GD2 T-cells.

Sarcomas are rare, mesenchymal tumors, representing about 10-15% of all childhood cancers. GD2 is a suitable target for chimeric antigen receptor (CAR) T-cell therapy due to its overexpression in several solid tumors. In this preclinical study, we investigated the potential use of iCasp9.

Rhabdomyosarcoma in children and young adults.

Rhabdomyosarcoma (RMS) is the most common soft tissue malignancy in childhood, accounting for 3% of all pediatric malignancies and 50% of all pediatric soft tissue sarcomas. In adolescents and young adults (AYA) however, RMS comprises only 6.5% of all soft tissue sarcomas.

[Mid-long term follow-up reports on head and neck rhabdomyosarcoma in children].

To analyze the clinical characteristics of children with head and neck rhabdomyosarcoma (RMS) and to summarize the mid-long term efficacy of Beijing Children's Hospital Rhabdomyosarcoma 2006 (BCH-RMS-2006) regimen and China Children's Cancer Group Rhabdomyosarcoma 2016 (CCCG-RMS-2016) regimen. A retrospective cohort study. Clinical data of 137 children with newly diagnosed head and neck RMS at Beijing Children's Hospital, Capital Medical University from March 2013 to December 2021 were collected.

Primary cutaneous rhabdomyosarcoma with EWSR1/FUS::TFCP2 fusion: four new cases with distinctive morphology, immunophenotypic, and genetic profile.

EWSR1/FUS::TFCP2-rearranged rhabdomyosarcoma (RMS) is a rare tumor with an aggressive clinical course, a predilection for craniofacial bones, spindled and/or epithelioid histomorphology, and positive immunohistochemistry (IHC) for epithelial and myogenic markers, along with variable ALK expression. Herein, we present four additional cases of primary cutaneous TFCP2-rearranged RMS. Notably, one tumor (case 1) displayed a varied pathological spectrum, initially presenting as a low-grade spindle cell neoplasm, but progressed into a high-grade spindle/epithelioid tumor.

Cytology of an Axillary Mass: A Rare Case.

This is an interesting case of rhabdomyosarcoma, presenting at an unusual site and diagnosed on FNAC. The smears depict the typical malignant small round cell morphology and tigroid background. It illustrates the role of cell block and immunohistochemistry as important ancillary adjuncts to morphology.

Upper Extremity Dedifferentiated Liposarcoma With an Osseus Component: An Uncommon Pathology.

Liposarcomas are the most common soft tissue sarcoma primarily originating in deep soft tissues and the retroperitoneum. Sarcoma classification includes atypical lipomatous tumor/well-differentiated liposarcoma (WDL) and dedifferentiated liposarcoma (DDL), myxoid liposarcoma, and pleomorphic liposarcoma. DDL is most prevalent in the retroperitoneum and often has two distinct components, a well-differentiated lipomatous component and a dedifferentiated nonlipomatous component that could be morphologically similar to malignant fibrous histiocytoma (MFH) or fibrosarcoma.

Maintenance therapy with trofosfamide, idarubicin and etoposide in patients with rhabdomyosarcoma and other high-risk soft tissue sarcomas (CWS-2007-HR): a multicentre, open-label, randomised controlled phase 3 trial.

Background: Rhabdomyosarcoma and other soft tissue sarcomas (STS) with high-risk features are still associated with an unsatisfactory outcome. We evaluated the efficacy of oral maintenance therapy added at the end of standard therapy in patients with high-risk rhabdomyosarcoma and STS.

Methods: CWS-2007-HR was a multicentre, open-label, randomised controlled, phase 3 trial done at 87 centers in 5 countries.

[Characteristics of the cytogenetic variants of alveolar rhabdomyosarcoma].

Unlabelled: Rhabdomyosarcomas (RMS) are one of the most common types of sarcomas in children and adolescents. The alveolar RMS subgroup is of particular interest because in some cases, the translocation of the and genes is combined with an amplification of the corresponding hybrid gene. According to literature data, the frequency of the translocation is 70-90% and the translocation 10-30%.

Desmoplastic Small Round Cell Tumors of the Gastrointestinal Tract.

Desmoplastic small round cell tumors (DSRCTs) of the gastrointestinal (GI) tract are a rare and highly aggressive variant of soft tissue sarcomas, predominantly affecting the abdominal region. These tumors are believed to originate from multipotent mesenchymal stem cells or primitive progenitor cells. They are composed of small round tumor cells associated with prominent stromal desmoplasia, polyphenotypic differentiation, and gene fusion.

Rhabdomyosarcoma of the Maxilla.

Rhabdomyosarcoma is the most common soft tissue sarcoma in children but is less frequent in adults, with the head and neck region as primary site. Magnetic resonance imaging (MRI) is the preferred diagnostic imaging tool, though its imaging characteristics are relatively non‑specific and overlap with other soft tissue sarcomas. The prognosis of rhabdomyosarcoma depends on the primary tumour site and size, with parameningeal head and neck localisations having a less favourable prognosis due to the higher risk of spread.

RMST: A long noncoding RNA involved in cancer and disease.

Long non-coding RNA Rhabdomyosarcoma 2-associated Transcript (RMST) is a crucial regulator in various biological processes, particularly in neurogenesis and cancer progression. This review summarizes current knowledge on structure, expression patterns, and functional roles across different organs and diseases of RMST. RMST exhibits tissue-specific expression, notably in brain tissues and vascular endothelial cells, and plays a significant role in neuronal differentiation through interaction with SRY-box 2 (SOX2).

Long-term Urinary and Sexual Outcomes in Pediatric Genitourinary Rhabdomyosarcoma Survivors: A Qualitative Study.

Purpose: Genitourinary rhabdomyosarcoma (GU-RMS) often requires multimodal therapy treatment including radiation, chemotherapy, and radical surgery for disease control. The long-term effects of the disease and associated treatments are unclear. We sought to investigate the long-term genitourinary quality of life for adult survivors of pediatric GU-RMS.

Malignant Phyllodes Tumor of the Breast With Multiple Cutaneous Metastasis Resembling Pleomorphic Rhabdomyosarcoma.

Malignant phyllodes tumor (PT) of the breast is a rare fibroepithelial neoplasm that shows variegated histomorphology and an aggressive clinical course. Cutaneous metastases are rare. A 68 year old woman presented with a palpable left breast mass identified on a routine breast exam.

Treatment of Pediatric, Adolescent, and Young Adult Patients With Fusion-Positive Alveolar Rhabdomyosarcoma Infiltrating Regional Lymph Nodes in the European CWS-2002P and RMS 2005 Studies and the Soft Tissue Sarcoma Registry.

Background: Patients with alveolar rhabdomyosarcoma (ARMS) with regional lymph node involvement (N1) are defined as "very-high-risk rhabdomyosarcoma" in Europe. Different chemotherapy regimens were used in European study protocols.

Methods: Patients with FOXO1 fusion-positive N1 ARMS registered in the CWS-2002P study, the EpSSG RMS 2005 study, and SoTiSaR were retrospectively investigated.